PB2645: HEMATURIA IN ADULT HAEMOPHILIC INDIVIDUALS: IS THE HEMAOPHILIA THE ONLY CAUSE OF HEMATURIA? A SINGLE CENTRE EXPERIENCE FOR A DECADE
Authors: Betül Kübra Tüzün, Zühal Demirci, Güray Saydam, Fahri Şahin
Journal: HemaSphere (2023)

Topic: 33. Bleeding disorders (congenital and acquired) Background: Hemophilia is an X-linked congenital bleeding disorder characterized by bleeding into the joint, soft tissue and intramuscular bleeding in hemophilic individuals. Less often from mucous membranes bleeding, central system bleeding, retropharyngeal/retroperitoneal bleeding, hematuria, and bleeding after surgical interventions/traumatic procedures are observed. Gynecological and obstetric bleeding may occur in carriers whose factor levels are between 30-50%. In the absence of a urethral clot, hematuria is expected in the presence of secondary benign causes. Aims: In this study, we have aimed to identify hematuria reasons in hemophilia patients who presented with macroscopic hematuria in the last decade in terms of aetiology and treatment outcomes. Methods:Results: For last decade, 44 hemophilic individuals have been evaluated due to macrospopic hematuria in Ege Adult Hemophilia and Thrombosis Center. Thirty-three of these individuals (%75) have the diagnosis of hemophilia A, 11 (25 %)have hemophilia B. The median age of the patients was 49 (20 to 85) years. When these patients were classified based on severity, 24 patients (54,5%) had severe, 14(31,8%) had moderate and 6 (13,6%) had mild hemopilia. Three individuals had inhibitör to factor 8 or 9.. While none of the patients described trauma, 1 patient had chronic NSAID use due to chronic inflammatory bowel disease. When these patients were consulted by Urology, urinary stone disease were detected in 13 patients;prostat/bladder cancers were diagnosed in 2 patients and, 2 patients had urinary tract infections. In remaining hemophilic patients, the etiology for macroscopic hematuria could not be detected. Surgical intervention was required in only 10 of the patients. When patients applied to our centre with macroscopic hematuria, 34 (77.3%) of patients were recieving prophylaxis and 10 (22.7) were recieving on demand treatment. Summary/Conclusion: The incidence of macroscopic and microscopic hematuria in hemophilia patients varies between 9% and 66%. Considering the etiopathogenesis of hematuria, it may be secondary to trauma, exercise, stress, use of vitamin D derivatives, spontaneous, NSAID use, as well as the first symptom of stones and urinary malignancies in the urinary system. In vast majority of patients with hemophilia and macroscopic hematuria, this situiation generally is accepted due to natural course of the underlying hemophilia, which rarely causes the macroscopic hematuria. The life expectancy of hemophilic individuals improved with prophylactic treatment practices and quality of life is almost similar to the normal population. As even a single episode of macroscopic hematuria is considered a significant risk factor for urologic disease, macroscopic hematuria requires the involvement of several specialists to investigate for detection of underlying disorder. Patients should be questioned in terms of hematuria at each follow-up and encouraged to apply to the outpatient clinic for hematuria that develops after conditions such as exercise and stress. Thus, it will facilitate the detection and management of preventable etiological factors. Keywords: Factor IX, Prophylaxis, Factor VIII, Hemophilia